Ricerc@Sapienza

Pulmonary arterial hypertension (PAH) is a clinical condition characterized by the presence of pre-capillary PH and pulmonary vascular resistance >3 UW, in the absence of other causes of pre-capillary PH [1]. Despite the availability over the past 20 years of multiple drugs, PAH prognosis is still poor with a median survival of 7 years [2]. As a rare disease the European Union encourages research and gives priority founding on this field [3]. Our Center is currently one of the two most important Italian centers for PAH [4]. Risk stratification in PAH is challenging; as in the most relevant and international registers, more than 80% of all patients with PAH are allocated at the intermediate and high risk with a variable treatment response and a different mortality rate [5-8]. Palpitation, syncope and sudden cardiac death are frequent findings in PAH patients and could be related to arrhythmias originating from the right heart [9-13]. As widely reported, arrhythmias confer a substantial risk of mortality and morbidity increasing risk of hospitalizations and clinical worsening. Nevertheless, cardiac arrhythmia in PAH has not yet well investigated and requires further investigation [14].
Aim of our study is to detect arrhythmias occurring in PAH patients with rhythm disorders in medical history, in order to better understand the pathophysiologic substrate. We¿ll conduct an observational and prospective study enrolling 30 consecutive PAH patients with palpitations, tachycardia or syncope in medical history. An electrophysiology study (EPS) will be performed during the routinely right heart catheterization. A voltage analysis and a conduction analysis will be performed in order to detect areas of electrical instability potentially determining arrhythmias. Researching on arrhythmias as a new feature on PAH and then finding out fatal or less severe arrhythmias can to better stratify the risk in PAH especially at the intermediate risk and helping the patients¿ management.